Context Germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene are responsible for a subset of familial isolated pituitary adenoma (FIPA) cases and sporadic pituitary neuroendocrine tumors (PitNETs). Objective To compare prospectively diagnosed AIP mutation-positive (AIPmut) PitNET patients with clinically presenting patients and to compare the clinical characteristics of AIPmut and AIPneg PitNET patients. Design 12-year prospective, observational study. Participants & Setting We studied probands and family members of FIPA kindreds and sporadic patients with disease onset ≤18 years or macroadenomas with onset ≤30 years (n = 1477). This was a collaborative study conducted at referral centers for pituitary diseases. Interventions & Outcome AIP testing and clinical screening for pituitary disease. Comparison of characteristics of prospectively diagnosed (n = 22) vs clinically presenting AIPmut PitNET patients (n = 145), and AIPmut (n = 167) vs AIPneg PitNET patients (n = 1310). Results Prospectively diagnosed AIPmut PitNET patients had smaller lesions with less suprasellar extension or cavernous sinus invasion and required fewer treatments with fewer operations and no radiotherapy compared with clinically presenting cases; there were fewer cases with active disease and hypopituitarism at last follow-up. When comparing AIPmut and AIPneg cases, AIPmut patients were more often males, younger, more often had GH excess, pituitary apoplexy, suprasellar extension, and more patients required multimodal therapy, including radiotherapy. AIPmut patients (n = 136) with GH excess were taller than AIPneg counterparts (n = 650). Conclusions Prospectively diagnosed AIPmut patients show better outcomes than clinically presenting cases, demonstrating the benefits of genetic and clinical screening. AIP-related pituitary disease has a wide spectrum ranging from aggressively growing lesions to stable or indolent disease course.

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Purpose: A large component of ambulatory oncology practice is management of telephone calls placed to and from the practice between outpatient appointments. However, scant information is available in the literature concerning oncology practice telephone calls. The specific aims of this study were to define telephone call volume and distribution in an active ambulatory oncology practice, describe the callers and reasons for the telephone calls, and examine any differences in call volume by practice characteristics.Methods: A descriptive retrospective design was used to analyze medical oncology and hematology telephone calls in a 4-month period. Two investigator-developed tools were validated and used to collect data on telephone call content and patient demographics. Results:The sample included 5,283 telephone calls to or from 1,486 different individuals. Individuals making and/or receiving more than one telephone call in the study period represented 56% of the telephone calls. For every 10 scheduled clinic appointments, seven telephone calls were received or made. The volume of telephone calls was significantly higher on Mondays and in the mornings. The reasons for high-volume telephone calls by diagnosis and frequency were identified, with 30% of telephone calls involving multiple reasons. Conclusion:The data demonstrate the impact of telephone calls on ambulatory oncology practice and highlight the complex and highly variable actions required to manage the telephone calls. The findings confirm and document specific practice patterns and identify subgroups that target repeat telephone calls as an area for improvement.